Thursday, December 10, 2009

Why is there a cooler on the table with all her pretty things, you ask?

to hold a cake shaped like a liver, ready for transplant!

Tuesday morning began with a scheduled appointment in Cardiology at PCMC. No big, sometimes sick kids have to be at the hospital on theirbirthdays among other holidays and special occasions-it's just part of the deal. We didn't expect to be there for long...then I'd have the day to finish her shopping for a MUCH to be celebrated 1st birthday.

I'd planned something a bit more intimate than Jonah's 1st birthday, mostly to avoid germs- but it just felt appropriate for it to be celebrated more privately with NaNa, PaPa, Grandma, Grandpa, Jonesy and Daddy.

While we were at the hospital we felt like London's tummy had looked bigger than the day before when she'd seen Dr. Book. So we were off to the Liver Clinic after meeting with the cardiologist. Dr. Book felt like another parasentisis (big needle poked into her tummy to drain fluid) would be the best course of treatment. So....

Down to Radiology. A few hour wait...decided to kill some time and grab something to eat. I am usually very good at keeping my emotions at bay until I am alone or at least in the privacy of my own home. But this day, my baby's birthday, I found myself crying uncontrollably in the cafeteria of all places. I couldn't stop, I was sobbing. Cute James, held my hand- not needing to say anything cause he knew just how I was feeling and exactly what I was thinking. I was in the hospital exactly a year before giving birth to my sweet baby girl. What an amazing day it was. We'd had a rough 2008- lost 2 dogs that we loved like children, a grandparent, my mom had major medical problems and our company had been owed a major sum of money. BUT putting all things in perspective were so grateful to have had a healthy baby girl and to put a bad year behind us. Like I've said before it felt like a trick to find out months later that she was ill and had been since conception.

This is definitely a road we never thought we'd be on. It's always someone else's child and you feel really bad for them, but you are so grateful that it's not your own, count your blessings. It's OK-that's what I use to think too, there's nothing wrong with that... I will say it has been horrible, it has been trying but I wouldn't change it for anything, cause we wouldn't have the-Lu-we-do. We wouldn't have seen the little miracles we have or appreciate things we didn't before. We have seen the very best in people, we have felt the love of our family, our friends, our church, our neighbors and even strangers. People I've never met before have enriched our lives and become our friends. We have been the beneficiaries of such graciousness, I almost feel guilty. We have been served by so many in various ways and it warms my heart. We have literally been showered with love and kindness. It really has been whats carried me at times.

After her procedure they found there wasn't as much floating fluid as expected but rather her intestines and other tissues in her gut are very inflamed. So, they will treat that now too. After a little recovery we were able to head home just in the nick of time to a birthday party that wasn't at all how I'd pictured it to be.

Luckily our sweet moms took over and made the most delicious soup, bread and salad. My Leslie made 2 cakes for Lulu and my Aunt Becky had ordered a third. Kathryn and Julia conspired about decorations and I came home to a lovely sight for sore eyes. Banners, balloons, candles, candy and a special hat for the birthday girl. Kathryn and I think alike cause she did everything as I would have, if not better.

It was a fabulous night. Tears shed, laughs had, the Spirit close by. Happy Birthday my sweet girl, thank you for fighting, thank you for teaching us and most of all thank you for coming into our family. I love you, everyone does.

*liver cake made by Leslie Smith...look at some of her other cakes:http://clsmithfamily.blogspot.com/2009/11/cakes.html she will be "in business" the start of the year. keep her in mind for birthdays, weddings or any special occasion, if she can make a liver cake that really impressed her docs-she can do anything!

I love the liver cake. I'm sorry you had to spend her special day at PCMC, but it looks like you ended the day on a good note. I agree with you about feeling tricked. After having my boys 2 months premature and having them spend a month in the NICU, I was overjoyed to have a healthy baby who I could take home only to find out two months later she was far from healthy. Our roads have not been easy but it is all worth it.Erica

More tears from me to you. I love you. I'm so sorry. And, I love these beautiful people that pick up the pieces while you are busy being #1 Mom to Little Lu. What lovelies. You deserve EVERY kindness you've received and then some. Continued prayers for you . . .

Happy Happy Birthday sweet Lulu! Oh how I wish I could snuggle her and take everything away! I question why such hard things happen to such amazing people like Lulu! I'm so sorry you had to spend her Birthday in the hospital and I'm sure your heart breaks every time they have to poke and prod at her...what a strong little girl you have! I LOVE LOVE the liver cake, so perfect and I'm so glad you have such great family and friends to make her Birthday just right. It's incredible that you can see the good in all the bad and that you wouldn't change it, our trials make us who we are! We love you guys and again and always Lulu and your sweet family is in our prayers!

LuLu's Family Blogs

Welcome to liverforlulu.blogspot

Meet beautiful, brave, tough-as-nails London (aka "LuLu"). She's been fighting Alpha 1 Anitrypsin Deficiency since birth. It's a genetic disease that causes liver disease and sometimes lung disease later in life.
She received a new, clean, healthy, pink, perfect, beautiful liver January 15th 2010 thanks to the generocity of a family willing to donate their childs organs to give life to others. She is LuLu's Angel.
We thank every person who takes the time to look at LuLu's blog. I am so happy that many now know what Alpha-1 is. I am grateful that some may change their veiws on organ donation because of London's life that she's been living with such dignity, strength and grace.
Feel free to email me, elizabethbarnum(at)hotmail(dot)com with questions or comments.

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Contributors

What is Alpha-1?

Alpha-1 Antitrypsin Deficiency (Alpha-1) is a condition that is passed from parents to their children through their genes.This condition may result in serious lung and/or liver disease at various ages in life. For each trait a person inherits, there are usually two genes and one gene comes from each parent. People with Alpha-1 have received two defective alpha-1 antitrypsin genes. One defective gene came from their mother and one from their father.There are many types of defective alpha-1 antitrypsin genes. The most common abnormal genes are called S and Z. Normal genes are called M. A person who does not have Alpha-1 will have two M genes (MM). People identified with Alpha-1 most commonly have two Z genes (ZZ). Current evidence suggests that there are about 100,000 people with Alpha-1 (ZZ) in the United States. Another deficient gene combination is SZ, although people with this gene combination are less likely to get lung or liver problems than those with two Z genes. (lulu is ZZ)

Alpha-1 occurs when there is a lack of a protein in the blood called alpha-1 antitrypsin or AAT that is produced by the liver. The main function of AAT is to protect the lungs from inflammation caused by infection and inhaled irritants such as tobacco smoke. The low level of AAT in the blood occurs because the AAT is abnormal and cannot be released from the liver at the normal rate. This leads to a build up of abnormal AAT in the liver that can cause liver disease and a decrease of AAT in the blood that can lead to lung disease.

Alpha-1 has been identified in virtually all populations and ethnic groups. It is estimated that about 1 in every 2,500 Americans have Alpha-1. Individuals with Alpha-1 may remain healthy throughout their lives. Early diagnosis and avoidance of risk factors such as cigarette smoking, can help prevent Alpha-1 from causing disease. An estimated 20 million people have one normal and one defective AAT gene. People with one normal gene and one defective gene (for example MZ) are called “carriers”. (james and liz) Carriers may pass the defective gene on to their children. Alpha-1 can lead to lung destruction and is often misdiagnosed as asthma or smoking-related Chronic Obstructive Pulmonary Disease (COPD). Alpha-1 can’t be diagnosed by symptoms or by a medical examination; you need to get a blood test to know for sure. Alpha-1 is the most common known risk factor for emphysema and COPD. About 3% of all people diagnosed with COPD may have undetected Alpha-1. Alpha-1 can lead to liver disease. The most serious liver diseases are cirrhosis and liver cancer. The World Health Organization (WHO), American Thoracic Society (ATS), and the European Respiratory Society (ERS) recommend that all individuals with COPD be tested for Alpha-1.

What does YOUR liver do for you?

How important is the liver?The liver is a vital organ. Without it we would not be able to live. Apart from the brain, it is the most complex organ in the body. It has a wide range of functions and acts very much like a factory. One of its most amazing features is its regenerative power. Some 9/10ths of the liver can be cut away and provided the remaining 1/10th is healthy, the liver will grow back to its original size.

The liver is a processorThe food that we eat passes into the stomach via the esophagus (gullet). The food is broken down by the action of the stomach and the small intestine. The nutrients from the food are then absorbed into the blood via the vessels in the walls of the intestines. These drain to veins which lead into the portal vein which carries the blood into the liver. The nutrients are processed in many different ways in the liver. This processing of the food is known as metabolism. The final products made are used by the body for energy and growth.

The liver is a manufacturerThe liver produces a number of different substances which are vital in order for healthy growth and development. For example: 1. The liver cells produce substances which assist with the clotting of the blood, e.g.Prothrombin. 2. The liver cells make proteins which are carried in the blood. These have many functions. Albumin, for example, helps to control the distribution of fluid in all parts of the body and transports many substances to and from the liver and kidneys to other parts of the body.

The liver is a storage depotThe liver stores energy in the form of glycogen (sugar). It also stores a number of other substances, e.g. copper, iron and vitamins.

The liver is a controllerThe liver plays an important part in controlling: 1. The correct level of many hormones within the body. 2. The blood sugar (glucose) levels. The liver stores glucose in the form of glycogen when there is too much glucose in the blood and releases it when the blood sugar level falls. 3. The amount of fluid the body retains and its distribution throughout the body. 4. The concentration of cholesterol which it converts into bile salts. 5. The action of many drugs

The liver is a filterThe liver removes many unwanted substances from the body. It plays an important role in controlling the harmful effects of some drugs and products of metabolism by changing them chemically before excreting them into the bile. Bilirubin (unconjugated or indirect) is produced when old red blood cells break down. The average life of a healthy red blood cell is 120 days. This bilirubin is carried in the blood stream, attached to albuimin, to the liver where it is altered into a different form (conjugated or direct bilirubin) which is then: 1. Excreted in the bile. 2. Transferred into the bile which passes via the bile ducts into the intestine and appears as brown pigment in the stool. Bile is continuously produced by the liver cells mainly by the action of bile salts. It collects in and passes out through the network of bile ducts into the intestine. Bile salts play an important role in the digestion of fats.

The liver is a defenderThe liver plays an important role in fighting many types of infection. In particular, protects the body against infection which arises in the gut. With so many different and complex functions, it is not surprising that more than 100 types of liver diseases have been identified in babies and children.